Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0034067 (emphysema)
 11,506 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Currently two genetic pulmonary disorders can be diagnosed before birth: alpha-1-antitrypsin deficiency and mucoviscidosis. For the latter there are two possible diagnostic techniques: first a study of the intestinal enzymes of the amniotic fluid, a reliable method only at the 18th week, and also a study of DNA markers (ADN) of the trophoblastic cells using molecular biological techniques: this can be performed from the 10th to 11th week of pregnancy but presupposes a family study in which there is already a subject suffering from the disorder. Foetal echocardiography enables various pulmonary abnormalities to be detected: pleural effusion, cyst, pulmonary hypoplasia and other disorders. This technique however has some limits, at least at present. Most often these severe malformations are revealed at birth such as respiratory distress or stillbirth. Adenomatous cystic malformations or congenital lobar emphysema, a posterior diaphragmatic hernia, and oesophageal atresia with oesophagotracheal fistula are the most frequent and are curable surgically.
Rev Mal Respir 1988
PMID:[Prenatal diagnosis and genetic diseases of the lung]. 326 Oct 24

26 patients presenting with panlobular emphysema between 1978 and 1984 were submitted to a thoracotomy for surgical resection of their bullae. Pre-operative and post-operative evaluations of the following were made: The level of dyspnoea in 5 grades. The FEV1 (VEMS) the forced vital capacity (CVF) the functional residual capacity (CRF) measured plethysmographically, the residual volume (VR), the total lung capacity (CPT). Arterial blood gases. The presence of radiological signs of compression on pulmonary angiography and on plain chest radiographs: an index of compression rated 0 to 6 was defined at the outset. The subjects were split into 3 groups based on their post-operative respiratory function: Group 1: no improvement (n = 7). Group 2: moderate improvement of dyspnoea (n = 11). Group 3: a big improvement in dyspnoea (n = 7). These three groups were homogenous as regards their preoperative respiratory function. On the other hand, the compression caused by the bullae based on the pre-operative radiological data was much greater (p 0.01) in the group of patients who improved. In addition when the index of compression was equal or superior to 3 (n = 14) all the patients were improved (n = 6). The comparison of pre- and post-operative values shows the following results: The change in blood gases was very limited in all groups; the ventilatory parameters were unchanged in group 1; on the other hand in group 2 and 3 one notes an overall improvement in pulmonary function. The size of the improvement however, was very variable from one individual to another.(ABSTRACT TRUNCATED AT 250 WORDS)
Rev Mal Respir 1986
PMID:[Evaluation of the efficacy of the surgical treatment of panlobular emphysema]. 378 24

We report an observation of pneumopericardium occurring, without iatrogenic trauma in the preceding days, during the course of an epidermoid bronchial carcinoma, treated for a year by radio- and chemotherapy. The pneumopericardium produced a very attenuated clinical picture and was resorbed without incidence. But the patient died a few weeks after an overwhelming haemoptysis. The radiological picture is very characteristic. The differential diagnosis is above all that of a pneumo-mediastinum. But in the latter case, the clear zone exceeds the level of the great vessels and subcutaneous cervical emphysema in generally very clear both clinically and radiologically.
Rev Mal Respir 1986
PMID:[Pneumopericardium complicating bronchial cancer]. 380 1

In recent years associations have been reported between male infertility and chronic pulmonary infections. These disorders include immotile-cilia syndrome, cystic fibrosis and Young's syndrome. For each disorder, case reports or small groups of patients have been described. However, it remains to be determined if these congenital disorders play a significant role in primary bronchiectasis (P.B). To do this, we compared the fertility rate of 35 patients (24 males, 11 females) with clinical and radiological evidence of P.B. and of 71 patients (54 males, 17 females) with secondary bronchiectasis (S.B) due to a defined chest disease (mainly tuberculosis and chronic bronchitis with emphysema). We abstracted the clinical records of this group of 106 patients, hospitalized between 1966 and 1983 for: age at the time of hospitalization, sex, diagnosis (P.B. vs. S.B.), association with sinusitis and situs inversus, socio-economic status, ethnic group, marital status and fertility (graded as 0 or 1+). The crude association between P.B. and infertility is strong (p greater than 0.10). However age may represent a confounding factor as patients with P.B. are younger (p less than 0.01) than patients with S.B., and more often single (p less than 0.01). Initially we restricted the analysis to married patients of both groups, and still got a significant association. Then we compared mean age in childless married people of both groups and observed a non-significant difference. Moreover, when we compared fertility in older patients, for example in people over 30 years old, the association still persists (p less than 0.03). In conclusion, the congenital disorders associating bronchiectasia and infertility must be looked for in the check up of P.B., all the more so as some can be cured of their infertility.
Rev Mal Respir 1985
PMID:[Epidemiological approach to the relationship between bronchiectasis and sterility]. 408 Dec 81

A deficiency of alpha 1 antiproteases is associated with severe and early emphysema. This emphysema can be experimentally produced in animals by endotracheal instillation of elastolytic proteases. Thus it would seem that emphysema is linked to an imbalance between proteases and antiproteases at the pulmonary level. This work studies the proteases, whose role in the genesis of emphysema is highly probable in view of the data in the literature (leukocyte elastase), disputed (macrophage elastase) or transitory (microbial elastases). We contrast the main agents capable of inhibiting these proteases (alpha 1 antiprotease and bronchial inhibitors) or of changing their activity (alpha 2 macroglobulins). The relative importance of these antiproteases is discussed in the light of studies made on bronchial secretions and bronchoalveolar lavage. These irritants may influence the protease - antiprotease equilibrium and favour the development of emphysema by increasing the proteases or decreasing the antiproteases. It appears that tobacco, as well as infection and anything which sets in motion the pulmonary phagocytes favour the liberation of leucocyte elastase. These attacks inactive the alpha 1 antiproteases in addition to the bronchial inhibitor. They may be recognized by a change in elastolytic and anti-elastolytic activity observed in bronchial secretions and in bronchoalveolar lavage (which is more disputed in the latter).
Rev Fr Mal Respir 1982
PMID:[Proteases, antiproteases and pulmonary emphysema]. 618 52

The mortality due to respiratory disease was studied in France between 1970 and 1974 as well as in seven other countries in the European Economic Community. The French results were presented as an index of mortality by cause of death, enabling a comparison of the mortality in different groups of the population. Data was supplied for 7 diagnostic groups defined according to List A of the International Classification of Diseases. The population studied consisted of men and women between 15 to 64 years, classified according to residence (urban or rural) and profession (agricultural worker or not). In addition the indices of mortality for farmers or agricultural employees were compared to men of the same social class, for the same period. The comparisons between the urban and the rural background revealed an excess mortality for respiratory tuberculosis, lung cancer, bronchitis, emphysema and asthma for those in urban areas. In the rural environment an excess mortality was noted for acute respiratory diseases in both men and women; this was also found comparing agricultural to non-agricultural workers. Lastly, if one compared agricultural and non-agricultural workers of the same social class, deaths due to acute and chronic respiratory infections were higher in the agricultural workers. These results show the relative importance already stressed in other studies, of acute respiratory diseases in agricultural workers.
Rev Fr Mal Respir 1983
PMID:[Mortality from respiratory diseases among agricultural and non-agricultural workers in France from 1970 to 1974]. 684 89

The definition and the classification of bullae are recalled and documented by some examples. Two types of bullae are opposed : bulla found in normal parenchyma and bulla associated with widespread emphysematous lesions. The biochemical and biological mechanisms of the emphysematous changes are briefly described, with a distinction of two different pathogenesis : on one hand, an inherited defect in connective tissue synthesis as in Marfan syndrome, and on the other hand, an unbalance between proteases and antiproteases. The pathologist's contribution to the classification of the surgical forms of emphysema is documented by an electron microscopic study of lung biopsy in some cases : two cases with bullae (a Marfan syndrome and an alpha 1 AT deficiency of the Pi Z phenotype) with diffuse panlobular emphysema; and 4 cases of bullae without radiological evidence of emphysema. The elastic fibres were modified only in the 2 first cases but were normal in the others. A follow up of such cases is needed.
Rev Fr Mal Respir 1980
PMID:[Morphological classification of surgical forms of bullous emphysema in adult. Role of electron microscopy (author's transl)]. 697 Mar 93

In emphysema, structural modifications (alveolar distension with destruction of the walls and of the pulmonary capillary bed) contribute to the understanding of pathophysiological disorders, such as increase of the static pulmonary volumes, loss of elastic recoil, impairment in gas transfer. In panlobular emphysema, the simultaneous destruction of ventilatory and circulatory structures explains the absence of shunt effect. The loss of elastic recoil is the main cause of maximal expiratory flow limitation and of bronchial obstruction which is qualified as "extrinsic". For a same degree of bronchial expiratory obstruction, the disorders of gas exchange and pulmonary haemodynamics are less pronounced in emphysema (panlobular) than in chronic bronchitis. The effects of voluminous bullae associated with diffuse emphysema are difficult to study and to demonstrate. The bullae accentuate the altered elastic properties of the emphysematous lung and contribute therefore to the expiratory flow limitation. The bullae are usually neither ventilated nor perfused; a dead-space effect is thus rare, but should be investigated. The effects of compression by the bullae of the adjacent parenchyma (excepting the haemodynamic effects) can be evaluated only by relatively sophisticated regional exploration methods, while taking into account morphological data (X-ray, angiography).
Rev Fr Mal Respir 1980
PMID:[Physiopathology of diffuse and bullous emphysema (author's transl)]. 700 18

The authors define small airways disease and then describe its anatomical characteristics. In the early stage bronchial inflammation is found along with arterial lesions. As the disease develops, there are multiple bronchial and vascular alterations, often accompanied by emphysema. The few existing studies of the morphology/lung function test correlation show a relationship between small airways disease and abnormal respiratory function tests. One of the aims was to find the most sensitive and specific tests using the smoker as a model. However, as only 25% of smokers develop clinical abnormalities, the main problem is probably not early screening, but finding the parameter with the highest prognostic value. This should be the FEVI, measured at about age 40. At a younger age, the information is still too incomplete. More studies are needed to justify early screening. Finally, the authors discuss the procedure to be followed when abnormalities are detected, and its possible consequences on lung function and behaviour.
Rev Fr Mal Respir 1981
PMID:[Small airways disease (author's transl)]. 703 67

Six healthy children, 5 boys and 1 girl aged from 13 months to 4 years had a severe measles bronchopneumonia. The end result is established chronic airflow obstruction. On a background of fairly severe respiratory failure there were paroxysms of breathlessness with fever, wheezes and cyanosis which were little affected by bronchodilators or steroids. The anatomical basis of this syndrome is before all else a bronchiolar obstruction, shown at autopsy in a child dying after four months of the illness. But also there is a disorder of the large bronchi with bronchiectasis and problems with ventilation centrally and/or peripherally. The major radiological signs are airways distension which is always clearly in keeping with emphysema and a thickening of the peri-bronchial walls which are clearly visible on tomography. Thus it appears that measles, as with other respiratory viruses, can lead to permanent sequelae in very young children. It is however possible that the respiratory sequelae may be due to associated viral infections, with adenovirus in particular. On the practical level, the occurrence of an early and severe measles pneumonia in a young child and its persistence with a hypoxia and a lowering of dynamic compliance requires a prolonged follow up and a guarded prognosis. Unfortunately the treatment of this type of chronic airflow obstruction is only symptomatic.
Rev Fr Mal Respir 1981
PMID:[Respiratory sequelae of severe measles (author's transl)]. 733 Apr 29


<< Previous 1 2 3 4 5 6 Next >>